Comparison of the investigational device exemption and post-approval trials of the Melody transcatheter pulmonary valve.

OBJECTIVE: We compared 5-year outcomes of transcatheter pulmonary valve (TPV) replacement with the Melody TPV in the post-approval study (PAS) and the investigational device exemption (IDE) trial. BACKGROUND: As a condition of approval of the Melody TPV after the IDE trial, the Food and Drug Administration required that a PAS be conducted to evaluate outcomes of TPV replacement in a "real-world" environment. The 5-year outcomes of the PAS have not been published, and the IDE and PAS trials have not been compared. METHODS: The cohorts comprised all patients catheterized and implanted at 5 IDE sites and 10 PAS sites. Differences in trial protocols were detailed. Time-related outcomes and valve-related adverse events were compared between the two trials with Kaplan-Meier curves and log-rank testing. RESULTS: 167 patients (median age, 19 years) were catheterized and 150 underwent TPV replacement in the IDE trial; 121 were catheterized (median age, 17 years) and 100 implanted in the PAS. Freedom from hemodynamic dysfunction (p = .61) or any reintervention (p = .74) over time did not differ between trials. Freedom from stent fracture (p = .003) and transcatheter reintervention (p = .010) were longer in PAS, whereas freedom from explant (p = .020) and TPV endocarditis (p = .007) were shorter. Clinically important adverse events (AEs) were reported in 14% of PAS and 7.2% of IDE patients (p = .056); the incidence of any particular event was low in both. CONCLUSIONS: Hemodynamic and time-related outcomes in the PAS and IDE trials were generally similar, confirming the effectiveness of the Melody TPV with real-world providers. There were few significant complications and limited power to identify important differences in AEs. The lack of major differences in outcomes between the two studies questions the usefulness of mandated costly post-approval studies as part of the regulatory process for Class III medical devices.

Use of the GORE® CARDIOFORM Septal Occluder for percutaneous closure of secundum atrial septal defects: Results of the multicenter U.S. IDE trial.

OBJECTIVE: To evaluate safety and efficacy of the GORE® CARDIOFORM Septal Occluder for percutaneous transcatheter closure of ostium secundum atrial septal defects. BACKGROUND: The GORE® CARDIOFORM septal occluder is a double-disc, low profile, soft, conformable device, with distinct advantages over the GORE® HELEX® Septal Occluder. METHODS: Subjects were enrolled in this single arm prospective study from 21 U.S. sites, and followed for 3 years. Primary endpoint was 6 month composite clinical success, comprised of technical success (implantation and retention of device), closure success (normalization of right heart size), no 30 day serious adverse events, and no device embolization or reintervention. Secondary endpoints included technical success, procedure success (technical success and ≤ 2 mm residual shunt at procedure conclusion), closure success (clinically insignificant or no residual shunt), and safety (freedom from 30 day serious adverse events and 6-month device events). RESULTS: Between October 2012 and May 2015, 50 pivotal and 350 continued access subjects underwent attempted transcatheter GORE® CARDIOFORM Septal Occluder implantation. Median age was 6.9 years, and mean static defect diameter 9.7 ± 3.1 mm. Device placement was achieved in 93.5% (374/400). Composite clinical success was 90.2% and clinical closure success was 98.8% at 6 months. Freedom from serious adverse events was 98.3% at 30 days, with no device embolizations or reinterventions through 6 months. CONCLUSIONS: The GORE® CARDIOFORM Septal Occluder has high composite clinical success and safety, performing well in defects ≤17 mm by stop flow stretched diameter. Single, multifenestrated, and deficient retroaortic tissue defects were well represented and successfully treated.

Saline Contrast Transesophageal Echocardiography in Fontan Patients: Assessment of the Presence, Type, and Size of Right to Left Shunts.

Right to left (R-L) shunts resulting in cyanosis or systemic embolization occur after the Fontan procedure. The primary modality of diagnosing these is angiography. Successful delineation of these shunts in Fontan patients using selective saline contrast transesophageal echocardiography (SCTEE) may allow for reduced radiation and contrast exposure. We hypothesized that SCTEE could accurately determine the presence, type, and semiquantitative shunt size of R-L shunts in Fontan patients. SCTEE was performed in Fontan patients undergoing angiography for clinical indications. Injections were performed in six sites: mid-Fontan, right and left pulmonary arteries, superior and inferior vena cavae, and innominate vein. R-L shunt size was subjectively graded as 0 = absent, 1 = small, and 2 = medium or large based on echo contrast density in the left atrium. SCTEE was compared to angiography. 33 patients with Fontan were studied with median age 15 years, median weight 50.1 kg, and median O2 saturation of 90% in the R-L shunt group and 95% in the no R-L shunt group. R-L shunt types included intracardiac shunts (ICS), veno-venous collaterals (VVCs), arteriovenous malformations (AVMs), and their combinations. SCTEE versus angiography results were the same for the presence, type, and size of R-L shunts in 79% (26/33). SCTEE identified shunts in 88% (29/33). Angiography identified shunts in 85% (28/33). Neither method missed any medium or large R-L shunts. SCTEE and angiography had similar accuracy. SCTEE accurately detected the presence, type, and size of R-L shunts in most Fontan patients in this study. This can be used to guide targeted angiography, reducing radiation exposure and contrast load.

CRISP: Catheterization RISk score for Pediatrics: A Report from the Congenital Cardiac Interventional Study Consortium (CCISC).

OBJECTIVES: We sought to develop a scoring system that predicts the risk of serious adverse events (SAE's) for individual pediatric patients undergoing cardiac catheterization procedures. BACKGROUND: Systematic assessment of risk of SAE in pediatric catheterization can be challenging in view of a wide variation in procedure and patient complexity as well as rapidly evolving technology. METHODS: A 10 component scoring system was originally developed based on expert consensus and review of the existing literature. Data from an international multi-institutional catheterization registry (CCISC) between 2008 and 2013 were used to validate this scoring system. In addition we used multivariate methods to further refine the original risk score to improve its predictive power of SAE's. RESULTS: Univariate analysis confirmed the strong correlation of each of the 10 components of the original risk score with SAE attributed to a pediatric cardiac catheterization (P < 0.001 for all variables). Multivariate analysis resulted in a modified risk score (CRISP) that corresponds to an increase in value of area under a receiver operating characteristic curve (AUC) from 0.715 to 0.741. CONCLUSION: The CRISP score predicts risk of occurrence of an SAE for individual patients undergoing pediatric cardiac catheterization procedures.

SCAI/CCAS/SPA Expert Consensus Statement for Anesthesia and Sedation Practice: Recommendations for Patients Undergoing Diagnostic and Therapeutic Procedures in the Pediatric and Congenital Cardiac Catheterization Laboratory.

Current practice of sedation and anesthesia for patients undergoing pediatric and congenital cardiac catheterization laboratory (PCCCL) procedures is known to vary among institutions, a multi-society expert panel with representatives from the Congenital Heart Disease Council of the Society for Cardiovascular Angiography and Interventions, the Society for Pediatric Anesthesia and the Congenital Cardiac Anesthesia Society was convened to evaluate the types of sedation and personnel necessary for procedures performed in the PCCCL. The goal of this panel was to provide practitioners and institutions performing these procedures with guidance consistent with national standards and to provide clinicians and institutions with consensus-based recommendations and the supporting references to encourage their application in quality improvement programs. Recommendations can neither encompass all clinical circumstances nor replace the judgment of individual clinicians in the management of each patient. The science of medicine is rooted in evidence, and the art of medicine is based on the application of this evidence to the individual patient. This expert consensus statement has adhered to these principles for optimal management of patients requiring sedation and anesthesia. What follows are recommendations for patient monitoring in the PCCCL regardless of whether minimal or no sedation is being used or general anesthesia is being provided by an anesthesiologist.

Management of hypoplastic left heart syndrome with intact atrial septum: a two-centre experience.

UNLABELLED: Introduction Hypoplastic left heart syndrome with an intact atrial septum is a poor predictor of outcomes. Prenatal assessment of pulmonary venous Doppler and emergent postnatal cardiac intervention may be associated with better outcomes. Materials and methods A retrospective review of all hypoplastic left heart syndrome patients in two centres over a 5-year period was performed. Group 1 included patients with adequate inter-atrial communication. Group 2 included patients with prenatal diagnosis with an intact atrial septum who had immediate transcatheter intervention. Group 3 included patients with intact atrial septum who were not prenatally diagnosed and underwent either delayed intervention or no intervention before stage 1 palliation. Primary outcome was survival up to stage 2 palliation. RESULTS: The incidence of hypoplastic left heart syndrome with a restrictive atrial communication was 11.2% (n=19 of 170). Overall survival to stage 2 or heart transplantation was 85% and 67% for Groups 1 and 2, respectively (n=129/151, n=8/12; p=0.03), and 0% (n=0/7) for Group 3. Survival benefits were observed between Groups 2 and 3 (p<0.001). Foetal pulmonary vein Doppler reverse/forward velocity time integral ratio of ⩾18% (sensitivity, 0.99, 95% CI, 0.58-1; specificity, 0.99, 95% CI, 0.96-1) was predictive of the need for emergent left atrial decompression. CONCLUSION: Using a multidisciplinary approach and foetal pulmonary vein Doppler, time-saving measures can be instituted by delivering prenatally diagnosed neonates with hypoplastic left heart syndrome with intact atrial septum close to the cardiac catheterisation suite where left atrial decompression can be performed quickly and safely that may improve survival.

Standardizing radiation dose reporting in the pediatric cardiac catheterization laboratory-a multicenter study by the CCISC (Congenital Cardiovascular Interventional Study Consortium).

OBJECTIVES: We examine normalized air Kerma area product (PKA ) by body weight (PKA /BW) as a reference value of radiation dose and benchmark PKA /BW in pediatric laboratories using a multicenter registry database. BACKGROUND: Reduction of radiation dose is an important quality improvement task in pediatric cardiac catheterization laboratories. Physicians need to agree on a standard method of reporting radiation dose that would allow comparisons to be made between operators and institutions. METHODS: This was a multicenter observational study of radiation dose in pediatric laboratories. Patient demographic, procedural and radiation data including fluoroscopic time and PKA (µGy m(2) ) were analyzed. PKA /BW was obtained by indexing PKA to body weight. RESULTS: A total of 8,267 pediatric catheterization procedures (age <18 years) were included from 16 institutions. The procedures consisted of diagnostic (n = 2,827), transplant right ventricular (RV) biopsy (n = 1,172), and interventional catheterizations (n = 4268). PKA correlated with body weight better than with age and best correlated with weight-fluoroscopic time product. PKA /BW showed consistent values across pediatric ages. Interventional catheterizations had the highest PKA /BW (50th, 75th, and 90th percentiles: 72, 151, and 281 µGy m(2) /kg), followed by diagnostic (59, 105, and 175 µGy m(2) /kg) and transplant RV biopsy (27, 79, and 114 µGy m(2) /kg). CONCLUSION: PKA /BW appeared to be the most reliable standard to report radiation dose across all procedure types and patient age. We recommend PKA /BW to be used as the standard unit in documenting radiation usage in pediatric laboratories and can be used to evaluate strategies to lower radiation dosage in pediatric patients undergoing cardiac catheterizations. © 2014 Wiley Periodicals, Inc.

Aortic Thrombosis and Subsequent Myocardial Infarction in a Previously Healthy 12-Year-Old Male.

Aortic thrombus formation in children is uncommon, particularly in an otherwise healthy pediatric patient. Thromboembolism of such thrombi resulting in subsequent ST-segment elevation myocardial infarction is, thus, exceedingly rare. (Level of Difficulty: Intermediate.).

Hemoptysis from an aorto-pulmonary collateral vessel in a four month old resolved by embolization.

Hemoptysis in the children is a rare but serious diagnosis and is even more uncommon in infancy. Mortality is reported and depends on associated illnesses, underlying etiology, and amount of bleeding. A 4-month-old patient presented with significant hemoptysis. Flexible bronchoscopy with differential lavage confirmed the presence and site of origin of hemoptysis. She was managed with cardiac catheterization for embolization of an aorto-pulmonary collateral vessel with immediate complete resolution and no further recurrences. This highlights the importance of cardiac catheterization to detect collateral vessels as a cause for hemoptysis at this age and its successful resolution following embolization. Pediatr Pulmonol. 2016; 51:E31-E33. © 2016 Wiley Periodicals, Inc.

Results of the U.S. Food and Drug Administration continued access clinical trial of the GORE HELEX septal occluder for secundum atrial septal defect.

OBJECTIVES: This report describes the immediate, 1-, and 5-year follow-up results of the U.S. Food and Drug Administration Continued Access clinical trial of the GORE HELEX Septal Occluder (W. L. Gore & Associates, Inc., Flagstaff, Arizona) for closure of secundum atrial septal defect. BACKGROUND: The trial was conducted between May 2003 and August 2006 to allow continued enrollment in a trial of the investigational device during review of data from the Pivotal Trial. Devices with hydrophilic coating on the expanded polytetrafluoroethylene to improve echocardiographic visualization were first used in this trial. METHODS: A total of 137 devices were implanted in 137 patients at 13 US institutions. Evaluations were scheduled at 1, 6, and 12 months for the initial trial and at 36 and 60 months for a later extension of the trial in those who consented to longer term evaluations. RESULTS: Twelve-month follow-up was completed on 122 of 126 patients with implantations, and 5-year follow-up on 83 of 95 patients who agreed to the trial extension. The overall clinical success rate was 96.7%, and the major adverse event rate 3.6%. Wire frame fractures were seen in 11.7% of patients with no clinical symptoms. A trivial, clinically insignificant leak was seen, or could not be ruled out, in 26.6% of patients at the 5-year evaluation, but no clinically significant leaks were seen. No patient experienced an erosion or sudden catastrophic event. CONCLUSIONS: The immediate, 1-, and 5-year follow-up outcomes of the Continued Access clinical trial continue to demonstrate that the GORE HELEX Septal Occluder is a safe and effective transcatheter occluder for repair of ostium secundum atrial septal defect.

Significance of low peak Doppler velocity in the proximal sano conduit in hypoplastic left heart syndrome.

BACKGROUND: The Sano modification of the Norwood operation is a well-established first step palliation for hypoplastic left heart syndrome (HLHS). Theoretically, the first point of resistance to pulmonary flow should be in the proximal Sano, generating high Doppler flow velocity. Paradoxically, however, some patients have low gradients in the proximal Sano conduit. The objective of this study was to determine the hemodynamic and anatomic significance of low proximal Sano Doppler flow velocity and its clinical implications. METHODS: Doppler-derived peak gradients in the proximal Sano conduits were measured in HLHS patients after Norwood-Sano surgery over a 4-year period and confirmed by cardiac catheterization within 2 to 4 weeks. Clinical outcomes of patients with proximal Sano gradients of 30 mm Hg or less (group 1) were compared with patients whose gradient was greater than 30 mm Hg (group 2). RESULTS: Of the 53 patients, 21 (40%) belonged to group 1. Patients in group 1 had smaller ostial right and left pulmonary artery (PA) diameter (3.2 ± 1.2 mm versus 4.5 ± 1.8 mm, p = 0.03; and 3.4 ± 1.2 mm versus 5.6 ± 2.1 mm, p = 0.01) when compared with patients in group 2. Patients (7 of 10) who needed either balloon angioplasty of a distal Sano or proximal branch PA were from group 1 (p = 0.01). Patients in group 1 had higher rates of unintended PA interventions (33% versus 9%) and complications (48% versus 16%) compared with group 2. CONCLUSIONS: Low peak Doppler flow velocity in the proximal Sano correlates with the presence of either distal Sano stenosis or proximal branch PA stenosis. These patients require close follow-up in the interstage period and may need earlier intervention.

One-year follow-up of the Melody transcatheter pulmonary valve multicenter post-approval study.

OBJECTIVES: This study sought to confirm that the short-term hemodynamic effectiveness of the Melody transcatheter pulmonary valve (TPV) (Medtronic, Inc., Minneapolis, Minnesota) achieved by real-world providers is equivalent to the historical results established in the initial 5-center Investigational Device Exemption trial. BACKGROUND: TPV replacement has been used to treat right ventricular outflow tract (RVOT) conduit dysfunction for >10 years. The Melody TPV received U.S. Food and Drug Administration approval in 2010 as a Humanitarian Use Device. METHODS: Patients with dysfunctional RVOT conduits were entered in this prospective, nonrandomized study at 10 centers. The primary endpoint was acceptable hemodynamic function at 6 months post-implantation, defined as a composite of RVOT echocardiographic mean gradient ≤30 mm Hg, pulmonary regurgitation less than moderate as measured by echocardiography, and freedom from conduit reintervention and reoperation. RESULTS: Cardiac catheterization was performed in 120 patients for potential implantation of the Melody TPV; of these, 100 patients were implanted, with a 98.0% procedural success rate. There were no procedure-related deaths. Acceptable hemodynamic function at 6 months was achieved in 96.7% of patients with evaluable data (87.9% of the entire implanted cohort), with results maintained through 1 year. No patient had moderate or severe pulmonary regurgitation after implantation. No patient required catheter reintervention in the first year after implantation, and 2 patients required reoperation for conduit replacement. The rate of freedom from TPV dysfunction was 96.9% at 1 year. CONCLUSIONS: This first prospective, real-world experience with the Melody TPV in the United States demonstrates continued high procedural success, excellent short-term TPV function, and low reintervention and reoperation rates at 1 year. (Melody Transcatheter Pulmonary Valve Post-Approval Study; NCT01186692).

Pre-existing left pulmonary artery stenosis and other anomalies associated with device occlusion of patent ductus arteriosus.

A retrospective analysis was performed on 200 consecutive patients who underwent cardiac catheterization for occlusion of Patent Ductus Arteriosus (PDA) at a single center by a single operator. Four significant anomalies were observed: pre-existing Left Pulmonary Artery (LPA) stenosis, left recurrent laryngeal nerve (LRLN) injury, electrocardiogram (EKG) changes, and aorto-pulmonary (AP) collateral arteries. The observation of pre-existing LPA stenosis, marked EKG changes, and permanent LRLN injury have not been previously reported. Incidence, etiology, and clinical significance of these anomalies are discussed with specific new recommendations for the prevention of LRLN injury and occlusion of AP collaterals.

Technical considerations for closing secundum atrial septal defect in the small child with the HELEX Septal Occluder via transhepatic access.

We describe closure of an atrial septal defect in a symptomatic 4.6 kg child with the HELEX Septal Occluder (W.L. Gore and Associates, Flagstaff, AZ) via transhepatic approach. Technical considerations are discussed.

Inadvertent stenting of patent ductus arteriosus with Amplatzer Vascular Plug.

We report a 12-month old patient who presented for murmur evaluation after percutaneous closure of type C patent ductus arteriosus (PDA) using a 10 mm Amplatzer Vascular Plug (AGA Medical Corporation, Golden Valley, MN) at an outside institution. Echocardiography revealed a large left-to-right shunt through the implanted device, inadvertently stenting the PDA instead of closing it. The patient underwent repeat catheterization with successful coil implantation within the Amplatzer Vascular Plug, completely eliminating the large residual ductal shunt. Although challenging, this case illustrates the technique of implanting coils within this occlusion device. This case also illustrates that occlusion of type C PDA utilizing the Amplatzer Vascular Plug may not only result in incomplete occlusion but also create a potentially worse clinical situation in which the PDA is stretched larger and stented open. Without consideration of simultaneous coil implantation within this device, use of the Amplatzer Vascular Plug might be contraindicated in type C PDA, because there may be no way to ensure successful closure by the Vascular Plug alone.

CRISP: catheterization RISk score for pediatrics: a report from the congenital cardiac interventional study consortium (CCISC)

OBJECTIVES:We sought to develop a scoring system that predicts the risk of serious adverse events (SAE's) for individual pediatric patients undergoing cardiac catheterization procedures. BACKGROUND: Systematic assessment of risk of SAE in pediatric catheterization can be challenging in view of a wide variation in procedure and patient complexity as well as rapidly evolving technology. METHODS: A 10 component scoring system was originally developed based on expert consensus and review of the existing literature. Data from an international multi-institutional catheterization registry (CCISC) between 2008 and 2013 were used to validate this scoring system. In addition we used multivariate methods to further refine the original risk score to improve its predictive power of SAE's. RESULTS: Univariate analysis confirmed the strong correlation of each of the 10 components of the original risk score with SAE attributed to a pediatric cardiac catheterization (P < 0.001 for all variables). Multivariate analysis resulted in a modified risk score (CRISP) that corresponds to an increase in value of area under a receiver operating characteristic curve (AUC) from 0.715 to 0.741. CONCLUSION: The CRISP score predicts risk of occurrence of an SAE for individual patients undergoing pediatric cardiac catheterization procedures.

Novel approach to the newborn with hypoplastic left heart syndrome and intact atrial septum.

A prenatally diagnosed fetus with hypoplastic left heart syndrome and intact atrial septum was delivered in the cardiac catheterization suite. Using radio frequency energy, a transseptal perforation of the thickened and intact atrial septum was immediately performed following transcatheter cannulation of the right atrium via the umbilical vein. Serial cutting balloon septostomies followed by static balloon septostomies resulted in effective left atrial decompression, atrial mixing, and optimal pulmonary and systemic perfusion. The child is now thriving after both stage I Norwood and bidirectional Glenn procedures.

Standardizing Radiation Dose Reporting in thePediatric Cardiac Catheterization Laboratory—AMulticenter Study by the CCISC (CongenitalCardiovascular Interventional Study Consortium)

We examine normalized air Kerma area product (PKA) by body weight(PKA/BW) as a reference value of radiation dose and benchmark PKA/BW in pediatriclaboratories using a multicenter registry database. Background: Reduction of radiationdose is an important quality improvement task in pediatric cardiac catheterizationlaboratories. Physicians need to agree on a standard method of reportingradiation dose that would allow comparisons to be made between operators andinstitutions. Methods: This was a multicenter observational study of radiation dosein pediatric laboratories. Patient demographic, procedural and radiation data includingfluoroscopic time and PKA (mGy m2) were analyzed. PKA/BW was obtained byindexing PKA to body weight. Results: A total of 8,267 pediatric catheterization procedures(age <18 years) were included from 16 institutions. The procedures consistedof diagnostic (n52,827), transplant right ventricular (RV) biopsy (n51,172),and interventional catheterizations (n54268). PKA correlated with body weight betterthan with age and best correlated with weight–fluoroscopic time product. PKA/BWshowed consistent values across pediatric ages. Interventional catheterizations hadthe highest PKA/BW (50th, 75th, and 90th percentiles: 72, 151, and 281 lGy m2/kg),followed by diagnostic (59, 105, and 175 lGy m2/kg) and transplant RV biopsy (27,79, and 114 lGy m2/kg). Conclusion: PKA/BW appeared to be the most reliablestandard to report radiation dose across all procedure types and patient age. We recommend PKA/BW to be used as the standard unit in documenting radiationusage in pediatric laboratories and can be used to evaluate strategies to lower radiationdosage in pediatric patients undergoing cardiac catheterizations.